Progression of limb apraxia in corticobasal syndrome: neuropychological and functional neuroimaging report of a case series.

The current study described the progression of limb apraxia in seven corticobasal syndrome patients through a comprehensive battery, including both gesture production tasks and conceptual tool/action knowledge tasks. The examination of the behavioral and neuroimaging (SPECT) data revealed two patient subgroups. One group consisted of patients with preserved conceptual tool/action knowledge, relatively mild gesture production and neuropsychological deficits with few significantly hypoperfused regions of interest. The other group consisted of those whose conceptual tool/action knowledge and general cognition eventually deteriorated and who were quite severely affected in their gesture production performance. These patients were characterized by bilateral hypoperfusion in parietal regions and in one case bilateral anterior cingulate regions.

FDG-PET patterns associated with ideomotor apraxia and imitation apraxia in patients with corticobasal syndrome.

INTRODUCTION: Apraxia is a core clinical feature of corticobasal syndrome (CBS). Among the subtypes of apraxia, ideomotor and imitation apraxia are frequently found in CBS. However, little is known about the brain networks that are characteristic of each apraxia subtype or their clinical implication. In this study, we used 18F-fluorodeoxyglucose positron emission tomography (FDG-PET) to explore the specific patterns of glucose hypometabolism that are characteristic of apraxia subtypes by focusing on ideomotor and imitation apraxia. METHODS: We compared the areas of glucose hypometabolism in the brains of 52 patients with CBS and 13 healthy controls, both as a whole and according to apraxia subtypes. In addition, we investigated the relationship between the apraxia subtypes and the clinical phenotype of CBS. RESULTS: In patients with CBS, common hypometabolism was observed in the frontal gyrus, precentral gyrus and caudate regardless of apraxia subtypes. In particular, ideomotor apraxia was associated with hypometabolism in the angular gyrus, while imitation apraxia was associated with hypometabolism in the posterior part including the postcentral gyrus, precuneus, and posterior cingulate gyrus. Patients who showed both ideomotor and imitation apraxia were more likely to show the typical features of CBS and progressive supranuclear palsy compared with patients showing only one type of apraxia. CONCLUSION: Group comparison analysis using FDG-PET revealed distinct pathways of ideomotor and imitation apraxia in CBS. These findings add to our understanding of the brain networks underlying apraxia in association with the clinical features of CBS.

Corticobasal syndrome with novel argyrophilic glial inclusions.

A 42-year-old, left-handed woman first noted impaired dexterity of the dominant hand, soon followed by dysarthria and cognitive decline. Over a 4-year period, she developed severe left-sided apraxia with eventual neglect of the left arm and progressive extrapyramidal signs. Cognitive testing showed progressive executive, visuospatial, fluency, and naming impairment with relative preservation of memory. Single-photon emission computed tomography demonstrated asymmetric right posterior frontal and superior parietal hypoperfusion. The clinical impression was corticobasal degeneration. At autopsy, severe atrophy was seen in the perirolandic and frontal regions. There was marked neuronal loss and gliosis in the posterior frontal and precentral regions and less severe pathology in prefrontal, temporal, and parietal areas. Mild to moderate gliosis and neuronal loss were also seen in the putamen, globus pallidus, subthalamic, and dentate nuclei. Gallyas silver stain revealed numerous inclusions adjacent to oligodendrocyte nuclei in white and gray matter of affected cortical and subcortical regions. The gracile inclusions were wavy, slender, and stained positively with antibodies to ubiquitin and alphaB-crystallin but not to microtubule-associated proteins (tau, MAP1B, MAP2), tubulin, neurofilaments, glial fibrillary acidic protein, or alpha-synuclein. The argyrophilic inclusions identified in this case are distinct from those previously described in neurodegenerative diseases.

Glucose hypometabolism in medial frontal cortex of patients with apraxia of lid opening.

PURPOSE: To determine whether regional cerebral functional abnormalities exist in patients with apraxia of lid opening (ALO). METHODS: Cerebral glucose metabolism was examined by positron emission tomography (PET) in 11 patients (8 women and 3 men, age 48-69 years); 10 with ALO accompanied by blepharospasm and 1 patient with pure ALO. Eleven normal volunteers (6 women and 5 men, age 45-66 years) were examined as controls. A comprehensive ophthalmological examination, magnetic resonance imaging (MRI), and PET were performed. The cerebral glucose metabolism was evaluated by the relative uptake of [fluorine-18]fluorodeoxyglucose by PET. The mean +/- two standard deviations of the normal controls was defined as the normal range for cerebral glucose metabolism. RESULTS: MRI revealed no particular lesion except for an infarction in the unilateral basal ganglia in two patients. Decreased glucose metabolism was observed in a wide area of the medial frontal lobe (six cases) and primary visual cortex (PVC) (four cases). Group multiple comparisons revealed a significant decrease ( P<0.0035) in the bilateral anterior cingulate gyrus, left supplementary motor area (SMA), and bilateral PVC. CONCLUSION: The results support the hypothesis that ALO is associated with hypofunction in the SMA and/or anterior cingulate gyrus.